Tetralogy of Fallot is a congenital heart condition that is associated with four specific defects inside the heart.

  • Ventricular septal defect – a hole in the heart between two lower chambers
  • Pulmonary stenosis – a tight pulmonary valve that does not open properly, which limits the amount of blood that gets to the lungs
  • Right ventricular hypertrophy – thickening of the wall on the right side of the heart, which happens because the ventricle is pumping against the stiff pulmonary valve
  • Overriding aorta – the aortic valve located directly over the ventricular septal defect

A common symptom of tetralogy of Fallot is when the baby's lips and tongue turn blue or purple due to low oxygen levels.

Other symptoms may include:

  • Sleepiness
  • Shortness of breath
  • Increased fussiness
  • In extreme cases, passing out

Read our Patient Guidebook on Tetralogy of Fallot

Our Patient Guidebooks provide additional information about the conditions we treat. They are not diagnoses and should never be used instead of medical advice.

Tetralogy of Fallot – Causes and Diagnosis

The majority of cases of tetralogy of Fallot have no known cause. 

Tetralogy of Fallot is diagnosed with the following tests: 

  • Cardiac catheterization
  • Cardiac MRI
  • Chest X-ray 
  • Comprehensive physical exam
  • Echocardiogram – ultrasound of the heart 
  • Pulse oximetry test – detects the amount of oxygen in the blood

Care Providers

The University of Maryland Children's Heart Program team that treats tetralogy of Fallot includes a pediatric cardiac surgeon, pediatric cardiologists, pediatric and neonatal intensive care doctors and nurses, pediatric cardiac anesthesiologists, cardiac operating room staff, cardiac nurses and many others with expertise in providing this specialized cardiac care. 
Our team of pediatric cardiologists also works closely with our clinical geneticists to explore all the possibilities if there is concern for a genetic syndrome. 

If tetralogy of Fallot is diagnosed while you are pregnant, you will see a maternal fetal medicine specialist who will treat you during your pregnancy. 

Treating Tetralogy of Fallot

Open heart surgery is needed to repair tetralogy of Fallot and typically occurs during the first few months of life. In some cases, the pulmonary valve will need to be removed completely. If this is the case, your child will require another surgery or a cardiac catheterization later in life.

Infants with a severe condition will require surgery soon after birth. In these cases, your baby will also need a complete tetralogy of Fallot repair during the first year of life.

The three main parts of the this repair include:

  • Closing the hole in your child’s heart (the ventricular septal defect or VSD)
  • Opening the right ventricle outflow tract
  • Repairing the pulmonary valve

Here at the University of Maryland Children's Hospital, we specialize in valve-sparing tetralogy of Fallot repair, which means we try very hard to keep your child’s pulmonary valve so that he/she will not need additional surgery later in life.

Patient Story

Read how our team of specialists treated Jade's tetralogy of Fallot.

Contact Us 

  • To learn more about Maternal Fetal Heart medicine, please call 410-328-3865.
  • To make an appointment with a Children's Heart Program physician, please call 410-328-4348.